AB0610 Development of Malignancy in Patients with ANCA-associated Vasculitis
Authors: M. Bektaş, B. Ince, S. Zarali, U. A. Gulseren, E. Ük, B. F. Agargun, D. Y. Guzey, Y. Yalçinkaya, B. Artim-Esen, A. Gül, M. Inanc
Journal: Annals of the Rheumatic Diseases (2022)

Background Patients (pts) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) reported to have an increased risk of malignancy compared to general population [1]. However, studies on risk factors for the development of malignancy in AAV pts are limited. Objectives We aimed to evaluate the frequency, clinical features and associated factors of malignancy in pts with AAV. Methods In this study, we retrospectively evaluated 287 pts with AAV. Thirty-three pts with EGPA and 14 pts with missing data were excluded. ANCA test was analysed based on immunofluorescence and/or Elisa results and divided into two serological groups; c-ANCA/PR3 + (positive) and p-ANCA/MPO + . Results Data of 240 pts (54.6 % female) were analysed. Mean age of diagnosis was 55.6±14 (range; 17-88) years and median disease duration was 67.5 (range; 3-255) months. Of those pts, 175 (73 %) had GPA and 65 (27 %) had MPA. ANCA results were available in 230 pts; 123 were c-ANCA/PR3 + (53.5 %), 85 were p-ANCA/MPO + (37 %) and 22 were ANCA negative (ANCA - ) (9.5 %). Kidney (75.8 %) and lower respiratory tract (74.4 %) were the most common organs involved but they did not differ according to presence of malignancy (9.4 % vs 5.1 %; p=0.4 and 9.4 % vs 5 % p=0.4 respectively.) Twenty-two malignancies were observed in 20 pts (8.3 %; eleven c-ANCA/PR3 + , three p-ANCA/MPO + and two ANCA negative pts). Lung and thyroid papillary cancer in three; bladder, prostate, breast and kidney in two; adrenal gland, oral squamous cavity and sarcoma of retroperitoneum in one patient, hematological (three myelodysplastic syndrome, one chronic lymphocytic leukemia and one lymphoma) in five pts. Six pts (30 %) had prior and/or concomitant AAV diagnosis. Development of malignancy did not differ according to age, gender, diagnosis, seropositivity (ANCA + vs ANCA - ) and ANCA subgroups (Table 1). There was no association between malignancy and cumulative dose of cyclophosphamide (CYC) or history of smoking (p=0.96 and p=0.2, respectively). In univariate analysis, malignancy was associated with presence of cardiovascular disease (CVD) (p=0.003 OR 11.7) and mortality (p=0.04 OR 4.6), higher BVAS score at baseline (p=0.049) and higher VDI score (p=0.02). Significant association was observed between malignancy with CVD (95 % CI 2.2-83 OR 13.4 p=0.005) and mortality (p=0.044 95 % CI 1.03-8.5 OR:2.95) in multivariate analysis. Table 1. Factors associated with malignancy in pts with AAV Univariate analysis Variables Malignancy + (n=20 ) Malignancy – (n=220 ) p value (OR ) Age (years) Ɨ 60±13.4 55.3±14 0.15 Gender (female) 10 (50) 121 (55) 0.7 Diagnosis * GPA 15 (8.6) 160 (91.4) 0.8 MPA 5 (7.7) 60 (92) ANCA status* c-ANCA/PR3 11 (9) 112 (91) 0.16 p-ANCA/MPO 3 (3.5) 82 (97) Cardiovascular disease* 8/38 (21) 10/199 (5) 0.003 (11.7 ) Cerebrovascular accident* 2/15 (13) 16/222 (7) 0.3 Coronary heart disease* 4/18 (22) 19/218 (9) 0.08 Avascular necrosis* 4/35 (11) 14/199 (7) 0.3 Venous thrombosis* 3/21 (14) 15/217 (7) 0.2 BVAS score at admission Ɨ 21.3±6 16.6±6.7 0.049 Smoking history (ever)* 6/53 (11) 8/156 (5) 0.2 Cumulative CYC dose (g) ĸ 5 (7.4) 4.5 (7.8) 0.96 VDI score ĸ 3.5 (4) 2 (2) 0.02 Relapse (n=230, %) 5/80 (6.3) 11/150 (7.3) 0.8 Remission at six months (n=160, %) 8/104 (7.7) 5/56 (9) 0.8 Mortality* 7 (35) 35 (16) 0.04 (4.6 ) OR: Odds ratio *(n, %) Ɨ mean± Std dev. ĸ median; IQR In survival analysis, mortality rate was higher in pts had malignancy compared to those without (Figure 1, p=0.035). Figure 1. Survival analysis of mortality according to presence of malignancy in pts with AAV. Log-Rank: p=0.035 Conclusion Significant proportion of pts with AAV developed malignancy in our cohort. Development of malignancy was associated with CVD, higher baseline BVAS and VDI scores. Our study also revealed lower survival rate in patients who developed malignancy. Further studies are needed to clarify risk factors for malignancy in patients with AAV. References [1]Wester et al. Curr Opin Rhe, 2018. 30 (1): p. 44-49 Disclosure of Interests None declared